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Dev Med Child Neurol. 2016 Jun;58(6):632-8. doi: 10.1111/dmcn.12984. Epub 2015 Dec 11.

Surgical fusion of early onset severe scoliosis increases survival in Rett syndrome: a cohort study.

Author information

1
Telethon Kids Institute, The University of Western Australia, Perth, WA, Australia.
2
School of Physiotherapy and Exercise Science, Curtin University, Perth, WA, Australia.
3
Department of Orthopaedics, Royal Children's Hospital, Melbourne, Vic., Australia.
4
Discipline of Genetic Medicine, The University of Sydney, Sydney, NSW, Australia.
5
Discipline of Paediatrics and Child Health, The Children's Hospital at Westmead, The University of Sydney, Sydney, NSW, Australia.
6
The Sydney Children's Hospitals Network (Westmead), Sydney, NSW, Australia.
7
Paediatric Spine Research Group, Lady Cilento Children's Hospital, Queensland University of Technology, Brisbane, Qld, Australia.
8
Department of Surgery, University of Queensland, Brisbane, Qld, Australia.
9
Discipline of Orthopaedics and Trauma, University of Adelaide, Adelaide, SA, Australia.
10
Department of Orthopaedic Surgery, Women's and Children's Hospital, Adelaide, SA, Australia.

Abstract

AIM:

Scoliosis is a common comorbidity in Rett syndrome and spinal fusion may be recommended if severe. We investigated the impact of spinal fusion on survival and risk of severe lower respiratory tract infection in Rett syndrome.

METHOD:

Data were ascertained from hospital medical records, the Australian Rett Syndrome Database, a longitudinal and population-based registry, and from the Australian Institute of Health and Welfare National Death Index database. Cox regression and generalized estimating equation models were used to estimate the effects of spinal surgery on survival and severe respiratory infection respectively in 140 females who developed severe scoliosis (Cobb angle ≥45°) before adulthood.

RESULTS:

After adjusting for mutation type and age of scoliosis onset, the rate of death was lower in the surgery group (hazard ratio [HR] 0.30, 95% confidence interval [CI] 0.12-0.74; p=0.009) compared to those without surgery. Rate of death was particularly reduced for those with early onset scoliosis (HR 0.17, 95% CI 0.06-0.52; p=0.002). There was some evidence to suggest that spinal fusion was associated with a reduction in risk of severe respiratory infection among those with early onset scoliosis (risk ratio 0.41, 95% CI 0.16-1.03; p=0.06).

INTERPRETATION:

With appropriate cautions, spinal fusion confers an advantage to life expectancy in Rett syndrome.

PMID:
26661519
DOI:
10.1111/dmcn.12984
[Indexed for MEDLINE]
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